A large number of neurodegenerative diseases in humans result from protein misfolding and
aggregation. Protein misfolding is believed to be the primary cause of Alzheimer's disease …

Integral membrane proteins are embedded in a watersolvated lipid bilayer that presents
contrasting features in an anisotropic, chemically heterogeneous environment. A …

Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in cftr, a gene encoding a
PKA-regulated Cl− channel. The most common mutation results in a deletion of …

Claudin-16 (Cldn16) is selectively expressed at tight junctions (TJs) of renal epithelial cells
of the thick ascending limb of Henle's loop, where it plays a central role in the reabsorption …

Endoplasmic reticulum (ER) stress is defined as an accumulation of unfolded proteins in the
endoplasmic reticulum. 4‐phenylbutyrate (4‐PBA) has been demonstrated to promote the …

A great deal of attention has been paid to so-called amyloid diseases, in which the proteins
responsible for the cell death and resultant diseases undergo conformational changes and …

Since the discovery of the first rhodopsin mutation that causes retinitis pigmentosa in 1990,
significant progresses have been made in elucidating the pathophysiology of diseases …

Mevalonate kinase deficiency (MKD) is an autosomal recessive autoinflammatory disorder
caused by mutations in the MVK gene resulting in deficient activity of mevalonate kinase …

An increasing number of studies indicate that low-molecular-weight compounds can help
correct conformational diseases by inhibiting the aggregation or enable the mutant proteins …

Most mutants of the cystic fibrosis transmembrane conductance regulator (CFTR) that cause
severe symptoms of cystic fibrosis do not reach the cell surface because they are defective …